A case of giant cholangioblastic cholangiocarcinoma (12 kg) with left hepatectomy and hilar reconstruction: a surgical and pathological challenge.

INTRODUCTION

Cholangioblastic cholangiocarcinoma is a rare variant of intrahepatic cholangiocarcinoma affecting young adults. Fewer than 30 cases have been reported. We describe a giant tumor in a 32-year-old man with morbid obesity (BMI of 54).

CASE PRESENTATION: The patient presented with a 34 cm mass, pain, persistent vomiting, inability to tolerate oral intake, and a 30 kg weight loss. Biopsy confirmed cholangioblastic intrahepatic cholangiocarcinoma. As mass-effect symptoms and malnutrition precluded initial systemic treatment, he underwent high-risk palliative cytoreduction. A fresh 12 kg tumor was removed by left hepatectomy. Inadvertent hilar stapling in severely distorted anatomy required immediate portal vein and hepatic artery reconstruction and Roux-en-Y hepaticojejunostomy. Hepatic inflow was compromised for approximately 75 minutes. Estimated blood loss was 20 L. He was discharged on postoperative day 14.

PATHOLOGY: The specimen measured 53 × 33.5 × 22 cm and weighed 7.914 kg after fixation. Histology showed extensive venous and lymphovascular invasion. An involved transection margin and residual intrahepatic and nodal disease constituted an R2 resection; six of nine nodes were metastatic (pT2, pN1).

DISCUSSION: This case illustrates the diagnostic, surgical, and anesthetic hazards of palliative resection for a giant hilar tumor, including the need for anatomical confirmation before hilar division and for preparedness for vascular reconstruction and massive hemorrhage.

CONCLUSION

Palliative cytoreduction relieved obstruction and enabled systemic therapy. The procedure was neither radical nor curative, and its potential symptomatic benefit must be balanced against substantial operative risk.